Characterisation of the treatment provided for children with unilateral hearing loss

Abstract

BackgroundChildren with permanent unilateral hearing loss (UHL) are an understudied population, with limited data to inform the guidelines on clinical management. There is a funding gap in healthcare provision for the children with UHL in the United Kingdom, where genetic screening, support services, and devices are not consistently provided or fully funded in all areas. They are a disparate population with regard to aetiology and their degree of hearing loss, and hence their device choice and use. Despite having one “good ear”, some children with UHL can have similar outcomes, socially, behaviourally, and academically, to children with bilateral hearing loss, highlighting the importance of understanding this population. In this longitudinal cohort study, we aimed to characterise the management of the children with UHL and the gaps in the support services that are provided for the children in Nottingham, United Kingdom.MethodsA cohort study was conducted collecting longitudinal data over 17 years (2002–2019) for 63 children with permanent congenital confirmed UHL in a large tertiary regional referral centre for hearing loss in Nottingham, United Kingdom. The cases of UHL include permanent congenital, conductive, mixed, or sensorineural hearing loss, and the degree of hearing loss ranges from mild to profound. The data were taken from their diagnostic auditory brainstem responses and their two most recent hearing assessments. Descriptors were recorded of the devices trialled and used and the diagnoses including aetiology of UHL, age of first fit, degree of hearing loss, when and which type of device was used, why a device was not used, the support services provided, concerns raised, and who raised them.ResultsMost children (45/63; 71%) trialled a device, and the remaining 18 children had no device trial on record. Most children (20/45; 44%) trialled a bone-conduction device, followed by contralateral routing of signal aid (15/45; 33%) and conventional hearing aids (9/45; 20%). Most children (36/45; 80%) who had a device indicated that they wore their device “all day” or every day in school. Few children (8/45; 18%) reported that they wore their device rarely, and the reasons for this included bullying (3/8), feedback from the device (2/8), and discomfort from the device (2/8). Only one child reported that the device was not helping with their hearing. The age that the children were first fitted with their hearing device varied a median of 2.5 years for hearing aids and bone-conduction devices and 7 years for a contralateral routing of signal aid. The length of time that the children had the device also varied widely (median of 26 months, range 3–135 months); the children had their bone-conduction hearing aid for the longest period of time (median of 32.5 months). There was a significant trend where more recent device fittings were happening for children at a younger age. Fifty-one children were referred by the paediatric audiologist to a support service, 72.5% (37/51) were subsequently followed up by the referred service with no issue, whilst the remaining 27.5% (14/51) encountered an issue leading to an unsuccessful provision of support. Overall, most children (65%, 41/63) had no reported concerns, and 28.5% (18/63) of the children went on to have a documented concern at some point during their audiological care: five with hearing aid difficulties, five with speech issues, four with no improvement in hearing, three facing self-image or bullying issues, and one case of a child struggling to interact socially with friends. Three of these children had not trialled a device. We documented every concern reported from the parents, clinicians, teachers of the deaf, and from the children themselves. Where concerns were raised, more than half (58.6%, 10/18) were by schools and teachers, the remaining four concerns were raised by the family, and further four concerns were raised by the children themselves.ConclusionTo discover what management will most benefit which children with permanent UHL, we first must characterise their treatment, their concerns, and the support services available for them. Despite the children with UHL being a highly disparate population—in terms of their aetiology, their device use, the degree of hearing loss, and the age at which they trial a device—the majority report they use their device mostly in school. In lieu of available data and in consideration of the devices that are available to them, it could be useful to support families and clinicians in understanding the devices which are most used and where they are used. Considering the reasons for cessation of regular device use counselling and support services would be vital to support the children with UHL.

Unilateral Sensorineural Hearing Loss in Children: Etiology, Audiological Characteristics, and Treatment

“…As a matter of fact, parents often perceive professionals’ hesitation and lack of conviction about the potential benefits of hearing aids in children with USNHL, as well as professionals’ tendency to minimize the importance of milder HL [ 38 ]. However, only one of our patients did not use a hearing aid due to lack of benefit after an adequate trial period, confirming that most children with UNSHL who have tried a hearing device continue to use it [ 9 , 33 ]. The subjective benefit of wearing a hearing aid was also indirectly proven by the high mean daily hearing aid use, with all but one of our patients using the device for at least 7 h per day.…”

“…Moreover, one in five children was diagnosed with unilateral vestibular hypofunction, reinforcing the need for routine vestibular and balance assessment in all pediatric patients with USNHL, as already suggested by previous studies [ 31 , 32 ]. Although several studies have demonstrated the negative consequences and long-term implications of uncorrected USNHL [ 9 , 13 , 14 , 15 , 16 ], less than half of our patients used a hearing device. The high percentage of non-users of hearing devices was largely attributed not only to aesthetic concerns due to social stigma, but also to parents’ lack of knowledge since this solution had never been proposed before by professionals.…”

“…However, it should be kept in mind that candidacy for unilateral cochlear implantation requires the careful assessment of imaging, audiological testing, duration of SNHL, age, comorbid conditions, psychological support, and parental motivation [ 18 , 43 ]. Alternative hearing technologies available for children with SSD include re-routing devices such as CROS and bone conduction devices, but these solutions cannot provide binaural input, which means they do not enhance sound localization [ 9 , 49 ]. Although these devices help attenuate the head shadow effect, they can also impair speech perception when the noise comes from the impaired ear’s side [ 9 ].…”

“…Alternative hearing technologies available for children with SSD include re-routing devices such as CROS and bone conduction devices, but these solutions cannot provide binaural input, which means they do not enhance sound localization [ 9 , 49 ]. Although these devices help attenuate the head shadow effect, they can also impair speech perception when the noise comes from the impaired ear’s side [ 9 ]. In particular, CROS hearing aids may have a negative effect in complex listening scenarios if patients are unable to manage the device according to the context, which is why these types of devices are generally not recommended under the age of 5 [ 50 ].…”

“…The prevalence of UHL increases with age; it is estimated that 7.2% of adults in the United States of America are affected by UHL [ 7 ]. The most common causes of unilateral sensorineural hearing loss (USNHL) include premature birth, congenital or postnatal infections (especially congenital cytomegalovirus and meningitis), structural anomalies (such as cochlear nerve hypoplasia/aplasia and enlarged vestibular aqueduct), genetic underpinnings, and temporal bone trauma [ 6 , 8 , 9 , 10 , 11 , 12 ]; however, in many cases, the etiology remains unknown [ 9 ]. A review by Lieu showed that UHL in children has a negative impact on speech–language development, cognition, and quality of life [ 13 ].…”